Evidence map›Paper›PMID 38769233›Full record

ArticleClinical autonomic research : official journal of the Clinical Autonomic Research Society2024

Cardiovascular autonomic failure in hereditary transthyretin amyloidosis and TTR carriers is an early and progressive disease marker.

Giacomo Chiaro, Claudia Stancanelli, Shiwen Koay, Ekawat Vichayanrat, Laura Sander, Gordon T Ingle, Patricia McNamara, Aisling S Carr, Ashutosh D Wechalekar, Carol J Whelan and 5 more

Abstract read
PubMed Publisher
In one paragraph

Article in Clinical autonomic research : official journal of the Clinical Autonomic Research Society, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

2 citing papers in PubMed.

  1. Article
  2. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

15 authors.

Giacomo ChiaroAutonomic Unit, National Hospital for Neurology and Neurosurgery, UCLH NHS Trust, London, UK.
Claudia StancanelliNemo SUD Clinical Centre, University Hospital "G. Martino", Messina, Italy.
Shiwen KoayAutonomic Unit, National Hospital for Neurology and Neurosurgery, UCLH NHS Trust, London, UK.
Ekawat VichayanratAutonomic Unit, National Hospital for Neurology and Neurosurgery, UCLH NHS Trust, London, UK.
Laura SanderAutonomic Unit, National Hospital for Neurology and Neurosurgery, UCLH NHS Trust, London, UK.
Gordon T IngleAutonomic Unit, National Hospital for Neurology and Neurosurgery, UCLH NHS Trust, London, UK.
Patricia McNamaraAutonomic Unit, National Hospital for Neurology and Neurosurgery, UCLH NHS Trust, London, UK.
Aisling S CarrCentre for Neuromuscular Diseases, Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology and the National Hospital for Neurology and Neurosurgery, London, UK.
Ashutosh D WechalekarNational Amyloidosis Centre, Division of Medicine, University College London, London, UK.
Carol J WhelanNational Amyloidosis Centre, Division of Medicine, University College London, London, UK.
Julian D GillmoreNational Amyloidosis Centre, Division of Medicine, University College London, London, UK.
Philip N HawkinsNational Amyloidosis Centre, Division of Medicine, University College London, London, UK.
Mary M ReillyCentre for Neuromuscular Diseases, Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology and the National Hospital for Neurology and Neurosurgery, London, UK.
Christopher J MathiasDepartment of Brain Repair and Rehabilitation, UCL Queen Square Institute of Neurology, University College London, London, UK.
Valeria IodiceAutonomic Unit, National Hospital for Neurology and Neurosurgery, UCLH NHS Trust, London, UK. v.iodice@ucl.ac.uk.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundThe cardiomyopathic and neuropathic phenotype of hereditary transthyretin amyloidosis are well recognized. Cardiovascular autonomic dysfunction is less systematically and objectively assessed.

methodsAutonomic and clinical features, quantitative cardiovascular autonomic function, and potential autonomic prognostic markers of disease progression were recorded in a cohort of individuals with hereditary transthyretin amyloidosis and in asymptomatic carriers of TTR variants at disease onset (T0) and at the time of the first quantitative autonomic assessment (T1). The severity of peripheral neuropathy and its progression was stratified with the polyneuropathy disability score.

resultsA total of 124 individuals were included (111 with a confirmed diagnosis of hereditary transthyretin amyloidosis, and 13 asymptomatic carriers of TTR variants). Symptoms of autonomic dysfunction were reported by 27% individuals at T0. Disease duration was 4.5 ± 4.0 years [mean ± standard deviation (SD)] at autonomic testing (T1). Symptoms of autonomic dysfunction were reported by 78% individuals at T1. Cardiovascular autonomic failure was detected by functional testing in 75% individuals and in 64% of TTR carriers. Progression rate from polyneuropathy disability stages I/II to III/IV seemed to be shorter for individuals with autonomic symptoms at onset [2.33 ± 0.56 versus 4.00 ± 0.69 years (mean ± SD)].

conclusionsCardiovascular autonomic dysfunction occurs early and frequently in individuals with hereditary transthyretin amyloidosis within 4.5 years from disease onset. Cardiovascular autonomic failure can be subclinical in individuals and asymptomatic carriers, and only detected with autonomic function testing, which should be considered a potential biomarker for early diagnosis and disease progression.

Indexed as

Amyloid Neuropathies, FamilialDisease ProgressionPrealbuminAdultAgedBiomarkersCohort StudiesFemaleHeterozygoteHumansMaleMiddle AgedBiomarkersPrealbuminTTR protein, humanATTRv amyloidosisAutonomic function testingCardiovascular autonomic failureDisease-modifying treatmentTTR carriers

Identifiers

PMID38769233

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.